HMBS Polyclonal Antibody, AbBy Fluor™ 594 Conjugated
Product Specifications
Background
PBGD, also designated hydroxymethylbilane synthase, is a cytoplasmic enzyme found in the heme synthesis pathway. PBGD belongs to the HMBS (hydroxymethylbilane synthase) family. Deficiency of PBGD causes errors in pyrrole metabolism, which in turn leads to an inherited autosomal disorder called acute intermittent porphyria (AIP) . AIP is characterized by acute attacks of neurological dysfunctions with hypertension, tachycardia, peripheral neurologic disturbances, abdominal pain and excessive amounts of aminolevulinic acid and porphobilinogen in the urine.
Synonyms
HEM3_HUMAN; HMBS; Hydroxymethylbilane synthase; PBG D; PBG-D; PBGD; Porphobilinogen deaminase; Pre uroporphyrinogen synthase; Pre-uroporphyrinogen synthase; UPS; Uroporphyrinogen I synthase; Uroporphyrinogen I synthetase.
Gene ID
3145
Host
Rabbit
Cross Reactivity
Human, Mouse, Rat
Immunogen
21-120/361
Target
HMBS
Clonality
Polyclonal
Isotype
IgG
Conjugation
AbBy Fluor™ 594
Source
KLH conjugated synthetic peptide derived from human HMBS/PBGD
Applications
IF
Purification
Purified by Protein A.
Excitation Emission
590nm/617nm
Concentration
1µg/µl
Dilution
IF (IF=1:50-200)
Buffer
Aqueous buffered solution containing 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Modification
Unmodified
Storage Conditions
Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
Gene ID URL
3145
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